Longitudinal follow-up of two patients with isolated paracentral acute middle maculopathy.

Objective: To describe longitudinal retinal changes in two cases of isolated paracentral acute middle maculopathy (PAMM). Case series: We reported two cases (64 and 67-year-old men) with isolated PAMM, who were followed-up for over 5 and 2.5 years, respectively. Both cases exhibited similar clinical natural histories. The first examinations took place several days after onset, with funduscopy showing that both cases exhibited paracentral scotoma with good visual acuity and small gray lesions, while optical coherence tomography (OCT) showed the presence of a hyperreflective band that ranged from the inner plexiform layer to the outer plexiform layer (OPL). The lesions became unremarkable within 1 month. The hyperreflective band also became unremarkable and was limited to the inner nuclear layer (INL) within 1 month, with the band disappearing within several months. Subsequently, OCT showed there was a thin and irregular INL and OPL, an excavated change of the inner retinal surface, along with outer nuclear layer (ONL) thickening. After several years, OCT angiography demonstrated normal flow of macular capillaries in the superficial capillary plexus, and decreased flow in the lesion with dilation of the capillaries around the area in the deep capillary plexus (DCP). Focal serous retinal detachment (SRD) occurred in one case after 4-5 years. Conclusion: Our findings indicated that long-term retinal changes in PAMM resulted in excavation of the inner retinal surface, INL thinning, ONL thickening and abnormal vasculature, especially in the DCP. Focal SRD may be a rare complication that can present at 4 years after onset.

Choroidal thickening and macular serous retinal detachment in pregnancy-induced hypertension.

OBJECTIVE: The purpose of this study was to report optical coherence tomography (OCT) and angiographic findings in a patient with pregnancy-induced hypertension (PIH). CASE REPORT: A 39-year-old woman, who was diagnosed with PIH, reported blurred and distorted vision at 5 days after an emergency cesarean delivery. OCT revealed a large serous retinal detachment (SRD) that included areas in the macula, along with an increased choroidal thickness noted in both eyes. Indocyanine green angiograms indicated delayed filling of the choroidal circulation in the early phase but choroidal hyperpermeability in the mid-phase. The SRD was gradually resolving without any treatment except for antihypertensive drugs. At 40 days after the initial examination, OCT revealed both the disappearance of the SRD and marked improvement of the choroidal thickening. CONCLUSION: Ophthalmologists need to be aware that PIH can cause choroidal ischemia, a breakdown of the outer blood-retinal barrier, and lead to the development of SRD.

Association between the major histocompatibility complex and clinical response to infliximab therapy in patients with Behçet uveitis.

PURPOSE: Our aim was to investigate whether major histocompatibility complex (MHC) polymorphisms are associated with response to infliximab therapy in Japanese patients with Behçet uveitis (BU). METHODS: We retrospectively reviewed 24 patients (17 men and seven women) treated with infliximab for BU. Of them, ten patients were genotyped as HLA A*2601, and nine as HLA B*5101. Therapeutic response levels in the two groups were compared based on ocular attacks and the Behçet disease ocular attack score 24 (BOS24) over 24 months of treatment. RESULTS: Mean frequencies of ocular attacks at 13-18 and 19-24 months after the start of treatment were significantly higher in the HLA A*2601 group (P = 0.0392 and 0.0177, respectively). Mean BOS24-6 M values for months 1-6, 7-12, 13-18, and 19-24 were also significantly higher in the HLA A*2601 group (P = 0.0459, 0.0150, 0.0394, and 0.0178, respectively). Shortening of the infusion interval was required in eight patients in the HLA A*2601 group but in one only in the HLA B*5101 group. Behçet-disease-related adverse events occurred in eight patients in the HLA A*2601 group and two in the HLA B*5101 group. Nonocular adverse events occurred in four patients in the HLA A*2601 group and none in the HLA B*5101 group. CONCLUSIONS: Although mean change from baseline in the number of ocular attack scores in the HLA A26 and HLA B51 groups seemed to be similar, the HLA-A26 group had a more severe disease course under infliximab therapy for ocular/extraocular involvement. These data suggest that response to infliximab therapy in Japanese patients with BU is partly due to genetic determinants in the HLA complex.

Presumed toxoplasmic central retinal artery occlusion and multifocal retinitis with perivascular sheathing.

Central retinal artery occlusion (CRAO) and multifocal retinitis with perivascular sheathing are rare in ocular toxoplasmosis. We report a case of toxoplasmic CRAO and multifocal retinitis with perivascular sheathing. A healthy 83-year-old male developed left panuveitis. Funduscopic examination of the left eye showed a swollen optic disc and sheathing of the retinal artery with a dense vitreous haze and a white retinal lesion. Serum anti-toxoplasma antibodies were positive in a latex agglutination assay. Vitrectomy was performed to improve visualization of the retinal lesions and for examination of causative microorganisms. A postoperative fundus examination revealed CRAO with optic disc involvement and multifocal retinitis with perivascular sheathing. Qualitative multiplex polymerase chain reaction detected the Toxoplasma gondii B1 gene in ocular fluid from both the aqueous and vitreous humor. The presumed diagnosis of ocular toxoplasmosis was made and treatment was started with prednisone and acetylspiramycin with subsequent improvement. Two months later, the patient developed active retinochoroiditis in the left eye. After 6 weeks of anti-toxoplasma therapy, the disease involuted. Retinal vascular occlusions and multifocal retinitis with perivascular sheathing are rare in toxoplasmosis. This is the first case report of toxoplasmic CRAO and multifocal retinitis with perivascular sheathing. The diagnosis of ocular toxoplasmosis should be considered in patients with retinal artery occlusions and multifocal retinitis with perivascular sheathing associated with inflammation.

Multifocal electroretinographic evaluation of macular function in acute posterior multifocal placoid pigment epitheliopathy.

BACKGROUND: In acute posterior multifocal placoid pigment epitheliopathy (APMPPE), little is known about the long-term outcome of electroretinographic macular function. The purpose of this study was to report 2-year follow-up results of multifocal electroretinography (mfERG) in a 26-year-old Japanese woman diagnosed with APMPPE. METHODS: Clinical and electrophysiological investigations of a single patient. RESULTS: Best-corrected visual acuity at initial examination was 1.5 and 0.5 in her right and left eyes, respectively. In addition to characteristic fundus lesions bilaterally, fluorescein angiography demonstrated diagnostic early blockage and late staining of the lesions. Optical coherence tomography revealed a hyperreflective spot (corresponding to the lesion) in the outer retinal layer in the right eye and intraretinal fluid in the left eye. On mfERG, the amplitudes were generally preserved, but markedly reduced amplitudes were detected in the central region of the left eye and in the paracentral region of the right eye. Five days later, visual acuity improved to 1.0, and the intraretinal fluid spontaneously disappeared without medication in the left eye. Light-to-dark ratios on electrooculography were 2.68 and 2.23 in the right and left eyes, respectively, both within the normal range. Two years later, visual acuity was 2.0 in both eyes, and ophthalmoscopically, there were neither retinal nor retinal pigment epithelial (RPE) abnormalities. mfERG revealed that the amplitudes were considerably improved (nearly normal level) in both eyes. CONCLUSIONS: The outcome suggests that longitudinal macular function in both visual acuity and mfERG may be favorable, unless areas of retinal or RPE alteration remain.

Subfoveal choroidal thickness in multiple evanescent white dot syndrome.

BACKGROUND: Multiple evanescent white dot syndrome (MEWDS) is an inflammation of the choriocapillaris, which typically presents with unilateral vision loss and is characterised by the presence of multiple yellow-white spots in the posterior pole to the midperipheral fundus. This study was conduced to evaluate subfoveal choroidal thickness between the acute and convalescent phases in two patients with MEWDS. METHODS: Two young female Japanese patients underwent a comprehensive ophthalmic examination, including slitlamp biomicroscopy, funduscopy and both fluorescein and indocyanine green angiographies. The subfoveal choroidal and central retinal thicknesses were measured using Cirrus high-definition spectral-domain optical coherence tomography. RESULTS: The two patients were diagnosed with unilateral MEWDS based on characteristic funduscopic and angiographic findings. The disrupted foveal inner segment-outer segment boundary line in the acute phase was restored in the convalescent phase in both patients. In the affected eye of Patient 1, the subfoveal choroidal thickness (337 µm) noted in the acute phase decreased to 249 µm at 133 days after the initial visit (convalescent phase). Similarly, the acute phase thickness (440 µm) in Patient 2 decreased to 358 µm at 133 days after the initial visit. The thickness in the asymptomatic opposite eye also decreased during the convalescent phase in both patients. In the acute phase, thickness in the affected eyes was greater than that in the opposite eyes in both patients. In contrast, central retinal thickness remained unchanged in both eyes during follow up in both patients. CONCLUSION: This is the first report to describe the relationship between subfoveal choroidal thickness and MEWDS. We found that the choroid was thicker in the acute phase than the convalescent phase in both the affected and opposite eyes of both patients, suggesting that an inflammatory reaction might occur in the choroidal stroma in addition to the choriocapillaris and might be bilateral rather than unilateral.